1f5l

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{{STRUCTURE_1f5l| PDB=1f5l | SCENE= }}
 
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===UROKINASE PLASMINOGEN ACTIVATOR B-CHAIN-AMILORIDE COMPLEX===
 
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{{ABSTRACT_PUBMED_10926521}}
 
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==Disease==
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==UROKINASE PLASMINOGEN ACTIVATOR B-CHAIN-AMILORIDE COMPLEX==
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[[http://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN]] Defects in PLAU are the cause of Quebec platelet disorder (QPD) [MIM:[http://omim.org/entry/601709 601709]]. QPD is an autosomal dominant bleeding disorder due to a gain-of-function defect in fibrinolysis. Although affected individuals do not exhibit systemic fibrinolysis, they show delayed onset bleeding after challenge, such as surgery. The hallmark of the disorder is markedly increased PLAU levels within platelets, which causes intraplatelet plasmin generation and secondary degradation of alpha-granule proteins.<ref>PMID:20007542</ref>
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<StructureSection load='1f5l' size='340' side='right'caption='[[1f5l]], [[Resolution|resolution]] 2.10&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[1f5l]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1F5L OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1F5L FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN]] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.1&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=AMR:3,5-DIAMINO-N-(AMINOIMINOMETHYL)-6-CHLOROPYRAZINECARBOXAMIDE'>AMR</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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==About this Structure==
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1f5l FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1f5l OCA], [https://pdbe.org/1f5l PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1f5l RCSB], [https://www.ebi.ac.uk/pdbsum/1f5l PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1f5l ProSAT]</span></td></tr>
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[[1f5l]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1F5L OCA].
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN] Defects in PLAU are the cause of Quebec platelet disorder (QPD) [MIM:[https://omim.org/entry/601709 601709]. QPD is an autosomal dominant bleeding disorder due to a gain-of-function defect in fibrinolysis. Although affected individuals do not exhibit systemic fibrinolysis, they show delayed onset bleeding after challenge, such as surgery. The hallmark of the disorder is markedly increased PLAU levels within platelets, which causes intraplatelet plasmin generation and secondary degradation of alpha-granule proteins.<ref>PMID:20007542</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/f5/1f5l_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1f5l ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
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*[[Urokinase|Urokinase]]
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*[[Urokinase 3D Structures|Urokinase 3D Structures]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:010926521</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: U-plasminogen activator]]
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[[Category: Large Structures]]
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[[Category: Jacob, U.]]
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[[Category: Jacob U]]
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[[Category: Karcher, A.]]
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[[Category: Karcher A]]
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[[Category: Schweinitz, A.]]
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[[Category: Schweinitz A]]
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[[Category: Sondermann, P.]]
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[[Category: Sondermann P]]
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[[Category: Sperl, S.]]
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[[Category: Sperl S]]
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[[Category: Sturzebecher, J.]]
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[[Category: Sturzebecher J]]
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[[Category: Zeslawska, E.]]
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[[Category: Zeslawska E]]
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[[Category: Human]]
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[[Category: Hydrolase]]
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[[Category: Inhibitor]]
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[[Category: Serine protease]]
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[[Category: Urokinase]]
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Current revision

UROKINASE PLASMINOGEN ACTIVATOR B-CHAIN-AMILORIDE COMPLEX

PDB ID 1f5l

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